Causes of secondary and tertiary adrenal insufficiency in adults

INTRODUCTION

Adrenal insufficiency can be caused by diseases of the adrenal gland (primary), interference with corticotropin (ACTH) secretion by the pituitary gland (secondary), or interference with corticotropin-releasing hormone (CRH) secretion by the hypothalamus (tertiary). This topic will review the major causes of the latter two disorders; the causes of primary adrenal insufficiency, and the clinical manifestations and approach to diagnosis are discussed separately. (See "Causes of primary adrenal insufficiency (Addison's disease)" and "Clinical manifestations of adrenal insufficiency in adults" and "Diagnosis of adrenal insufficiency in adults".)

 

SECONDARY ADRENAL INSUFFICIENCY

Any process that involves the pituitary and interferes with ACTH secretion can cause secondary adrenal insufficiency. The ACTH deficiency may be isolated, or occur in conjunction with other pituitary hormone deficiencies (panhypopituitarism).

 

Panhypopituitarism — Pituitary tissue can be destroyed and hormone secretion reduced by large pituitary tumors or craniopharyngiomas, infectious diseases such as tuberculosis or histoplasmosis, infiltrative diseases, lymphocytic hypophysitis, head trauma, and large intracranial artery aneurysms. Pituitary infarction can occur at the time of delivery if excessive blood is lost and hypotension occurs (Sheehan's syndrome), and hemorrhage may occur into a pituitary tumor (pituitary apoplexy). Pituitary metastases are frequently (about 5 percent) found in patients with disseminated cancer at autopsy; however, these metastases rarely reduce hormone secretion [1]. (See "Causes of hypopituitarism".)

 

ACTH deficiency due to genetic pituitary abnormalities is rare. ACTH and cortisol deficiency have been described in patients with multiple pituitary hormone deficiencies due to mutations in the PROP-1 (Prophet of Pit-1) gene, even though PROP-1 is not expressed in corticotropes. The onset of cortisol deficiency, which may be severe, ranges from childhood to late adulthood [2-5]. Mutations in other transcription factors involved in early pituitary development (HESX1, LHX4) also can result in variable degrees of hypopituitarism that include ACTH deficiency [6,7]. (See "Causes of hypopituitarism".)

 

Isolated ACTH deficiency — Isolated ACTH deficiency is a rare disorder [8]. The defect is probably at the pituitary level because there is no ACTH secretory response to CRH or vasopressin, as there usually is in hypothalamic disorders [9-11]. Occasional patients may have hypothyroxinemia and hyperprolactinemia that are corrected with glucocorticoid replacement [12,13].

 

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The Emerging Role of Robotics in Adrenal Surgery

The Emerging Role of Robotics in Adrenal Surgery

Journal
Current Urology Reports

Publisher
Current Medicine Group LLC

ISSN
1527-2737 (Print) 1534-6285 (Online)

DOI
10.1007/s11934-009-0079-7

Subject Group
Medicine

Accepted

PDF (133.6 KB) | HTML | Free Preview

Authors

James S. Rosoff¹ , Brandon J. Otto¹ , Joseph J. Del Pizzo¹

¹New York-Presbyterian Hospital Department of Urology, Weill Cornell Medical Center 525 East 68th Street, Starr 900 New York NY 10065 USA

Abstract

Abstract  Robotic surgery is being performed more frequently for a variety of urologic procedures. Since the first robotic adrenalectomy less than a decade ago, this modality has gained increased acceptance in the urologic community and has been employed with increased frequency in minimally invasive centers. This review evaluates the current literature on robotic adrenalectomy, its indications, as well as its advantages and limitations compared with other forms of surgical management of adrenal pathology.

Keywords

Adrenalectomy, Robotics, Minimally invasive

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From http://www.metapress.com/content/kn5x5nr334561875/

Incidentally-discovered Adrenal Masses

Author: Milton D Gross

Specialty: Radiology, Nuclear Medicine, Endocrinology
Institution: Division of Nuclear Medicine, Department of Radiology, University of Michigan
City: Ann Arbor; State/Province: Michigan; Postal Code: 48105; Country: United States

Author: Melvyn Korobkin

Specialty: Radiology
Institution: Division of Abdominal Imaging, Department of Radiology, University of Michigan Medical Center
City: Ann Arbor; State/Province: Michigan; Country: United States

 

Author: Wessam Bou-Assaly

Specialty: Radiology
Institution: Department of Radiology, University of Michigan Medical Center
City: Ann Arbor; State/Province: Michigan; Country: United States
Institution: Radiology Service, Department of Veterans Affairs Health System
City: Ann Arbor; State/Province: Michigan; Country: United States

Author: Domenico Rubello

Specialty: Radiology
Institution: Department of Nuclear Medicine, PET Center, and Medical Physics and Radiology, Santa Maria della Misericordia Hospital
City: Rovigo; Country: Italy

 

Abstract: Unanticipated adrenal masses are frequently encountered in modern, high resolution diagnostic imaging. Most often, these masses are benign adrenal adenomas, but when detected they necessitate a clinical evaluation sufficient to exclude subclinical endocrine disease, primary adrenal cancer, and remote metastases to the adrenal glands from other malignancies. These "incidentally-discovered" adrenal masses or so-called "adrenal incidentalomas" can be further evaluated with CT, MRI, and nuclear medicine imaging techniques. A substantial literature supports the use of each of these modalities to non-invasively characterize these neoplasms that have been considered by some as a 'disease' by modern imaging technology.

 

Introduction

Over the last 3 decades the more widespread use of high-resolution CT and MRI have seen the frequent, “incidental” discovery of clinically unsuspected masses of the adrenal glands. Detection of these masses raises the specter of malignancy and forces further diagnostic evaluation to determine their etiology and to distinguish benign masses from those that would substantially change the clinical approach to the patient.

 

In patients undergoing CT for reasons other than adrenal disease, adrenal masses are incidentally discovered in 0.4-4.5% (Kloos et al., 1995). The majority of adrenal masses, ranging from 7% to 94%, are benign and hormonally non-functional, while in patients with malignancy the incidence of unsuspected adrenal masses ranges from 7% to 68% (Kloos et al., 1995). Metastases from other cancers to the adrenal occur in up to 20% of patients without previously diagnosed cancers and in almost 75 % of patients harboring non-adrenal primary malignancies (Kloos et al., 1995) (Table 1).

 

The incidence of clinically unsuspected adrenal masses increases with age greater than 30 years, regardless of gender. Clinically silent, hormonally active adrenal masses include tumors that produce hormones from all of the functional zones of the adrenal cortex and the adrenal medulla. As a result it is important that once discovered, and prior to any further imaging, a biochemical evaluation sufficient to exclude pheochromocytoma, normokalemic primary aldosteronism, and subclinical hypercortisolism be done as these conditions are optimally managed by surgical removal of the offending tumor (Young, 2007).

 

Methods of Adrenal Gland Imaging

Computed tomography is the most common imaging modality that identifies unsuspected adrenal masses in the evaluation of the chest and upper abdomen for diseases unrelated to the adrenal glands (Gross at al., 2005). Both CT and MRI can be used to differentiate normal from abnormal adrenal glands and MRI has been used to characterize incidentally discovered adrenal masses in clinical situations where CT is non-diagnostic (Gross et al., 2009) (Table 2).

 

Computed tomography and magnetic resonance imaging

 

Contemporary CT scanners using slice thicknesses of 3-5 mm will reliably depict the adrenal glands in virtually all patients. Comparison of non-contrast to contrast-enhanced CT can be used to distinguish adrenal masses and for evaluating retention and patterns of contrast washout as a means to differentiate adenomas from other adrenal neoplasms (Boland et al., 2008). MRI can be used to advantage in the evaluation of normal adrenal glands and adrenal masses. Gradient-echo, chemical shift MRI with in- and opposed-phrase imaging, and dynamic contrast-enhanced MRI detect lipid content in adrenal adenomas and, when combined with chemical shift ratios, depict the adrenal glands with an efficacy similar to CT (Dunnick et al., 2002). Hybrid scanners marrying CT, and perhaps in the near future MRI, with positron emission tomography (PET) and single photon emission tomography (SPECT) employing radiopharmaceuticals designed to target unique aspects of adrenal gland function(s) offer direct combination of anatomic and functional information that enhances diagnosis and differentiation of benign from malignant adrenal lesions (Gross et al., 2009).

 

Adrenal scintigraphy

The radiocholesterol analogs, ¹³¹I-6β-iodomethyl-19-norcholesterol (NP-59) and selenium-75-6β-iodomethyl-19-norcholesterol (Scintadren®) were some of the first successful radiopharmaceuticals used to image the adrenal cortex (Gross et al., 2007). Radiolabeled inhibitors of enzymes responsible for adrenal steroid hormone biosynthesis like the 11β-hydroxylase inhibitor, carbon-11-metomidate (¹¹C-MTO), can also be used to image neoplasms of adrenocortical origin. Other radiolabeled substrates depict metabolic processes such as the radio-fluorine-labeled ¹⁸F-fluorodeoxyglucose (FDG) to identify primary adrenal neoplasms and metastases to the adrenals, while ¹¹C-labeled acetate and ¹¹C- and ¹⁸F-labeled choline have been used to depict adrenal adenomas (Gross et al., 2007).

Radiolabeled metaiodobenzylguanidine (MIBG) has been used to identify the adrenal medulla and localize neoplasms of adrenomedullary origin by exploiting norepinephrine-re-uptake mechanisms into the catecholamine storage vesicles of adrenergic tissues.

 

Hydroxyephedrine, an analog of norepinephrine, can be labeled with ¹¹C or ¹⁸F, and is transported into adrenergic nerve terminals by these same mechanisms. The list of catecholamine-based PET radiopharmaceuticals used to image the adrenal medulla, related tissues, and tumors of sympathomedullary origin now includes ¹¹C-epinephrine, ¹¹C- or ¹⁸F-hydroxyepinephrine, ¹⁸F-fluorodopamine (FDA), and ¹⁸F-fluorodihydroxyphenylalanine (¹⁸F-DOPA) (Shulkin et al., 2006). Alternatively, somatostatin receptor uptake of specific imaging agents can be used to image tissues that express somatostatin receptors, like the adrenal medulla and many other tissues and neoplasms. Octreotide, a long-acting somatostatin antagonist labeled with a variety of radioisotopes (¹¹¹Indium, ¹²³Iodine, ^99mTechnetium for SPECT, and ⁶⁸Gallium for PET imaging) can be used to accurately localize and stage primary and metastatic sympathomedullary tumors (Gross et al., 2007).

 

Imaging Incidentally-discovered Adrenal Masses

The differential diagnostic list of incidentally-discovered adrenal masses is long and a description of all but the most common is outside the scope of this short review. An extensive medical literature is available that describes the imaging characteristics of adrenal masses (Gross et al., 2005).

 

Adrenal adenoma

Adrenal adenomas are depicted on CT with density values expressed as Hounsfield units (HU) that are similar to normal adrenal tissues and depending upon their lipid content may express HU values similar to tissue water density (Korobkin et al., 1996). Furthermore, adenomas demonstrate increasing density, so-called “enhancement,” after intravenous CT contrast with more rapid loss of contrast — “washout” as compared to adrenal metastases (Korobkin et al., 1998). Like CT, MRI characteristics of adenomas are also similar to normal adrenal tissues. Signal intensity of adenomas is low on T2-weighted MR imaging sequences, but there is overlap (20-30%) with the signal intensity of metastases to the adrenal. Chemical-shift imaging is useful to depict tissue lipid content and is often employed to differentiate adenomas from metastases to the adrenal (Dunnick et al., 2002).

 

Pheochromocytoma

Pheochromocytomas are often clinically silent and present on high resolution anatomic imaging as incidentally-discovered adrenal masses. These neoplasms show enhancement with intravenous contrast agents in a fashion similar to malignant adrenal masses and occasionally may mimic contrast washout characteristics of benign adrenal adenomas. The imaging uncertainty posed by pheochromocytomas demands a biochemical evaluation sufficient to exclude catecholamine hypersecretion in suspect patients. Concerns over the potential of intravenous contrast-induction of hypertensive crisis have recently been allayed with nonionic contrast agents. Like CT, pheochromocytomas are usually hyperintense on T2-weighted imaging; however, there is overlap and about 30% are hypointense on T2-weighted imaging sequences (Blake et al., 2004).

 

Adrenal carcinoma

Adrenal carcinoma is a rare neoplasm (~ 2/10⁶ patients) that usually presents late in the course of disease. Adrenal carcinoma is often imaged as a large, heterogeneous abdominal mass with central necrosis, calcification, and tumor venous extension on contrast enhanced CT (Gross et al., 2009). On MRI these neoplasms are hyperintense on both T1- and T2-weighted imaging sequences from intra-tumor hemorrhage and central necrosis.

 

Adrenal metastases

Adrenal glands are common sites of remote metastases from cancers of the lung, breast, and melanoma. Adrenal metastases can be unilateral, bilateral, and variable in size. CT and MRI are nonspecific. Small metastases are often homogeneous on contrast CT or MRI, while large metastases are heterogeneous as a result of intra-metastasis necrosis and hemorrhage.

 

Distinguishing benign from malignant adrenal masses

Figure 1. An adrenal mass (arrow) is identified on CT (panel A) with characteristics of an adenoma with a non-contrast CT density (panel B) of 9.8 HU.

 

CT can be used to distinguish adrenal adenomas from metastases since most adenomas demonstrate non-contrast densities that are lower than metastases to the adrenal. The highest diagnostic efficacy for diagnosis of adrenal adenomas is obtained by selecting a threshold density value of 10 HU on non-contrast CT, as HU values for adenomas and adrenal hyperplasia are typically lower than those of metastases to the adrenal and pheochromocytomas (Figures 1 and 2).

 

Chemical-shift MRI also differentiates adrenal adenomas from metastases. By exploiting the different resonant frequencies of hydrogen in tissue water and lipid molecules, chemical-shift MRI detects a loss of signal intensity of tissues with high lipid and water content. Using gradient-echo imaging techniques, signal intensity loss on opposed-phase as compared to in-phase images differentiates tissues that contain lipids with high sensitivity and specificity. As lipids are common components of adrenal adenomas that are usually absent in metastases, this difference in lipid content can be exploited for the differentiation of adenomas vs. metastases to the adrenals (Figures 3 and 4).

Figure 2. Abdominal CT of bilateral adrenal masses (arrows) shows persistent enhancement of the right adrenal gland after intravenous contrast at 1.5 (panel B) and 5 min (panel C) after contrast injection in a pattern compatible with metastases to the adrenals.

 

In a combined histological CT and MRI study of adrenal adenomas there was an inverse relationship between lipid-containing cells and unenhanced CT, and a positive correlation with the relative change in signal intensity on opposed-phase MRI confirming that non-contrast CT and chemical-shift MRI might not necessarily complement each other in the evaluation of adrenal adenomas (Outwater et al., 1996). Contrast-enhanced CT images of the adrenals are obtained about 1 minute after a bolus intravenous injection of contrast and at this time point the attenuation values of adenomas and metastases are nearly identical (Figure 2B and Figure 5C). However, over time adenomas demonstrate loss of contrast enhancement and attenuation values on contrast enhanced CT of < 30-40 HU at 10-15 minutes post contrast injection can be used to distinguish, with few exceptions, adenomas from other adrenal masses (Gross el al., 2009).

 

Figure 3. MRI of a right adrenal nodule (arrows) demonstrates post-contrast enhancement on T1-weighted imaging (panel C) and displays loss of signal on out-of-phase (panel B) compared to in-phase imaging (panel A) confirming the mass as an adrenal adenoma.

 

Adrenal gland contrast washout as a percentage of initial enhancement is another parameter that can be used to identify adenomas from other adrenal masses. Adrenal adenomas have characteristic washout percentages of ~ 60% at 15 minutes post contrast administration corresponding to a sensitivity of 88% and a specificity of 96% for diagnosis of adenoma (Korobkin et al., 1998) (Figure 5). Further, so-called “lipid-poor” adenomas with unenhanced CT attenuation of >10 HU have contrast enhancement washout values that are nearly identical to those of lipid-rich adenomas confirming the value of differential washout as a means to identify adrenal adenomas from other adrenal masses (Caoili et al., 2000).

 

Distinguishing adrenal adenoma from carcinoma

Figure 4. MRI of a right adrenal mass demonstrates heterogeneous post-contrast enhancement (panel C) with no signal loss on out-of-phase imaging (panel B) vs. in-phase imaging (panel A) confirming the mass to be an adrenal metastasis.

 

Any adrenal mass larger than 5 to 6 cm in diameter is considered suspicious for an adrenocortical carcinoma. Large, non-secreting pheochromocytomas, adrenal carcinoma, or adrenal metastases may be indistinguishable by CT and MRI. Size criteria for resection of adrenal masses varies widely from 3.5 to 6 cm, and while there is controversy concerning the approach to smaller masses there is general agreement that masses > 6 cm, regardless of their imaging characteristics, should be removed.

 

Scintigraphy of adrenal masses

There are a host of scintigraphic imaging agents, with each targeting a unique characteristic of adrenal gland function that can be used to assess the etiology of incidentally-discovered adrenal masses. Adrenal adenomas can be depicted with iodocholesterol with positive and negative predictive values of 89% and 100%, respectively, and the lateralization of tracer uptake to one gland may be predictive of the development of future functional autonomy.

 

Metaiodbenzylguanidine images masses of adrenomedulla origin with positive and negative predictive values of 83% and 100%, respectively, while ¹⁸F-FDG separated benign from malignant adrenal lesions with 100% sensitivity and specificity (Maurea et al., 2001). As a result, if functional imaging is to be used to evaluate adrenal masses in patients with no history of cancer, radiocholesterol scintigraphy should be the first imaging procedure to identify the most common incidentally-discovered adrenal mass, a benign adrenal adenoma, followed by MIBG to identify clinically silent pheochromocytomas. Should MIBG be non-diagnostic, ¹⁸F-FDG is to be used to identify a potentially malignant adrenal mass or remote metastasis to the adrenal. Conversely, in patients with a prior history of cancer, ¹⁸F-FDG would be the most optimal first imaging study in the search for metastatic disease to the adrenal followed in sequence by iodocholesterol and MIBG.

Figure 5. A left adrenal gland mass (arrow) with a density of 1.5 HU on non-contrast CT (panels A and B) increases after intravenous contrast administration to 50 HU on early post-contrast enhanced images (panel C), and falls to 4 HU on the delayed images (panel D). The calculated washout of 95% is compatible with a lipid rich, adrenal adenoma.

 

¹⁸F-FDG-PET can differentiate benign from malignant adrenal lesions and metastases to the adrenal (Figure 6). In a study of 150 patients with adrenal masses, ¹⁸F-FDG-PET had a sensitivity of 98.5% and a specificity of 92% at a standardized uptake value (SUV) threshold of 3.1 and with the addition of CT to FDG-PET specificity increased to 98% with all masses correctly characterized as benign or malignant (Metser et al., 2006). While in 50 patients with known or suspected malignancy, ¹⁸F-FDG-PET had a sensitivity of 100%, a specificity of 94%, and an accuracy of 96%, and by using the liver as a ‘normal’ comparison tissue it improved the sensitivity of ¹⁸F-FDG for detecting malignant adrenal lesions without sacrificing sensitivity (Yun et al., 2001). More recently ¹⁸F-FDG-PET was shown in a prospective multicenter trial to accurately differentiate adrenocortical adenomas from adrenal carcinomas and in particular a subgroup of masses with indeterminate findings on CT (Groussain et al., 2009).

 

In comparative studies of the 11β-hydroxylase inhibitor, ¹¹C-MTO, and ¹⁸F-FDG in incidentally-discovered masses, ¹¹C-MTO identified lesions of adrenocortical origin with the highest SUV in adrenocortical carcinoma, followed by hypersecretory adrenal cortical adenomas and non-hypersecretory adenomas, although ¹¹C-MTO-PET did not distinguish benign adrenocortical neoplasms from adrenocortical carcinoma (Hennings et al., 2006). ¹⁸F-FDG can be also used to image pheochromocytomas and adrenocortical carcinoma and to differentiate these neoplasms from other non-hypersecreting and most hypersecreting adrenal adenomas.

Figure 6. PET/CT scans of adrenal masses (white arrows). An ¹⁸F-FDG scan (panel A) shows no appreciable tracer accumulation in a left adrenal mass seen on CT (panel B) compatible with an adrenal adenoma. An adrenal metastasis from lung cancer (black arrows) is depicted on an ¹⁸F-FDG scan (panel C) as an intense focus of tracer uptake in a small left adrenal mass on CT (panel D).

 

Pheochromocytomas can be reliably imaged with ¹²³I- or ¹³¹I-MIBG with PET/CT using ¹⁸F-dopamine or ¹⁸F-DOPA (Shulkin et al., 2006) (Figure 7). In a comparison of the efficacy of ¹⁸F-FDA, ¹²³I-MIBG, and ¹¹¹In-octreotide, ¹⁸F-FDA demonstrated the highest sensitivity in the localization of intra-adrenal and metastatic pheochromocytomas, followed by ¹²³I-MIBG and ¹¹¹In-octreotide for intra-adrenal pheochromocytoma, while the efficacy of ¹⁸F-FDA and ¹²³I-MIBG were equivalent (Ilias et al., 2008). If catecholamine analogs are not successful in imaging, the neoplasm may have experienced malignant dedifferentiation, and ¹⁸F-FDG or ¹¹¹In-octreotide can be used to localize metastases and guide subsequent therapy.

 

Summary

Incidentally-discovered adrenal masses are commonly encountered in modern high-resolution imaging. The list of differential diagnostic possibilities of incidentally-discovered adrenal masses is large, but fortunately, most are benign and non-hyperfunctioning adenomas. The first consideration in the evaluation of an incidentally-discovered adrenal mass is its functional status and should include a biochemical evaluation sufficient to exclude clinically-silent endocrine disease. CT, MRI, and scintigraphy can be used to characterize incidentally-discovered adrenal masses and distinguish adrenal adenomas from metastases to the adrenals and other adrenal neoplasms. An understanding of the imaging techniques used to distinguish benign from malignant and other incidentally-discovered adrenal masses speeds diagnosis, optimizes therapy, and decreases costs in the evaluation of these neoplasms.

Figure 7. A small, left adrenal mass (arrow) on CT (panel A) demonstrates intense ¹⁸F-DOPA uptake (panel B) in a patient with hypertension and a prior negative ¹²³I-MIBG scan who was later shown to have elevated catecholamines compatible with a pheochromocytoma.

 

References

Blake MA. Kalra MK. Maher MM. Sahani DV. Sweeney AT. Mueller PR. Hahn PF. Boland GW. Pheochromocytoma: an imaging chameleon. Radiographics 24(Suppl 1):S87-99, 2004.

Boland GW. Blake MA. Hahn PF. Mayo-Smith WW. Incidental adrenal lesions: principles, techniques, and algorithms for imaging characterization. Radiology 249(3):756-75, 2008.

Caoili EM. Korobkin M. Francis IR. Cohan RH. Dunnick NR. Delayed enhanced CT of lipid-poor adrenal adenomas. Am J Roentgenol 175(5):1411-5, 2000.

Dunnick NR and Korobkin M. Imaging of adrenal incidentalomas: current status. Am J Roentgenol 179(3):559-68, 2002.

Gross MD, Korobkin M, Hussain H, Cho KJ, Bui C. “Adrenal Gland Imaging” Chapter 126. Endocrinology, 5^th Edition. JL. Jameson and LJ. Degroot, Eds. W.B. Saunders, Philadelphia, USA, pp. 2425-2453, 2005.

Gross MD, Avram A, Fig LM, Rubello D. Contemporary adrenal scintigraphy. European J Nucl Med Mol Imaging 34:547-57, 2007.

Gross MD, Korobkin M, Bou Assaly W, Dwamena B, Djekidel M. Contemporary imaging of incidentally discovered adrenal masses. Nat Rev Urol 6:363-73, 2009.

Groussin L, Bonardel G, Silvera S, Tissier F, Coste J, Abiven G, Libe R, Bienvenu M, Alberini JL, Salenave S, Bouchard P, et al. ¹⁸F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. J Clin Endocrinol Metab 94(5):1713-22, 2009.

Hennings J, Lindhe O, Bergstrom M, Langstrom B, Sundin A, Hellman P. [¹¹C]metomidate positron emission tomography of adrenocortical tumors in correlation with histopathological findings. J Clin Endocrinol Metab 91(4):1410-4, 2006.

Ilias I, Chen CC, Carrasquillo JA, Whatley M, Ling A, Lazurova I, Adams KT, Perera S, Pacak K. Comparison of 6-¹⁸F-fluorodopamine PET with ¹²³I metaiodobenzylguanidine and ¹¹¹in-pentetreotide scintigraphy in localization of nonmetastatic and metastatic pheochromocytoma. J Nucl Med 49(10):1613-9, 2008.

Kloos RT, Gross MD, Francis IR, Korobkin M, Shapiro B. Incidentally discovered adrenal masses. Endocr Rev 16(4):460-84, 1995.

Korobkin M, Brodeur FJ, Francis IR, Quint LE, Dunnick NR, Londy F. CT time-attenuation washout curves of adrenal adenomas and nonadenomas. Am J Roentgenol 170(3):747-52, 1998.

Korobkin M, Giordano TJ, Brodeur FJ, Francis IR, Siegelman ES, Quint LE, Dunnick NR, Heiken JP, Wang HH. Adrenal adenomas: relationship between histologic lipid and CT and MR findings. Radiology 200(3):743-7, 1996.

Maurea S, Klain M, Mainolfi C, Ziviello M, Salvatore M. The diagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med 42(6):884-92, 2001.

Metser U, Miller E, Lerman H, Lievshitz G, Avital S, Even-Sapir E. ¹⁸F-FDG PET/CT in the evaluation of adrenal masses. J Nucl Med 47(1):32-7, 2006.

Outwater EK, Siegelman ES, Huang AB, Birnbaum BA. Adrenal masses: correlation between CT attenuation value and chemical shift ratio at MR imaging with in-phase and opposed-phase sequences. Radiology 200(3):749-52, 1996.

Shulkin BL, Ilias I, Sisson JC, Pacak K. Current trends in functional imaging of pheochromocytomas and paragangliomas. Ann N Y Acad Sci 1073:374-82, 2006.

Yun M, Kim W, Alnafisi N, Lacorte L, Jang S, Alavi A. 18F-FDG PET in characterizing adrenal lesions detected on CT or MRI. J Nucl Med 42(12):1795-9, 2001.

[Discovery Medicine, Volume 9, Number 44, January 2010. Pre-published.]

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From http://www.discoverymedicine.com/Milton-D-Gross/2010/01/04/incidentally-discovered-adrenal-masses/

Laparoscopic management of adrenal lesions larger than 5 cm in diameter - Abstract

Monday, 11 January 2010

Muljibhai Patel Urological Hospital, Nadiad, Gujarat, India.

Laparoscopic adrenalectomy remains a controversial procedure for large tumors. The incidence of adrenocortical carcinoma increases and technical difficulty of adrenalectomy increases as the size increases. We examined the outcome and complications of laparoscopic adrenalectomy for such lesions.

 

Twenty-nine patients underwent laparoscopic adrenalectomy, of whom 19 had tumors larger than 5 cm in diameter, having a median tumor size of 7.0 cm. They were compared with patients whose adrenal tumors were smaller than 5 cm.

 

Patients with small tumors (< 5 cm) had a significantly shorter median operative time of 90 minutes as compared to 145 minutes in those with large tumors (> 5 cm). There was no significant difference in the median hemoglobin drop (1.05 g/dL versus 1.30 g/dL), time for starting oral intake (24 hours in both groups) or hospital stay (3.5 days versus 4.0 days) between patients with small and large tumors, respectively. There were no intra-operative complications except for 1 incidence of supraventricular tachycardia in a patient with a large pheochromocytoma. There were no major complications seen in any of the patients and no open conversions. Histopathology of large tumors revealed 16 benign tumors (8 pheochromocytomas, 4 adenomas, 2 ganglioneuromas, 1 pseudocyst, and 1 myelolipoma) and 3 malignancies, of which 1 was primary adrenocortical carcinoma and 2 were metastatic renal cell carcinoma.

 

In experienced hands, laparoscopic adrenalectomy is safe and feasible for large functioning adrenal tumors. Large adrenal tumors suspicious of harboring malignancy with no peri-adrenal involvement can be tackled laparoscopically.

 

Written by:
Sharma R, Ganpule A, Veeramani M, Sabnis RB, Desai M.   Are you the author?

 

Reference:
Urol J. 2009 Fall;6(4):254-9.

PubMed Abstract
PMID:20027553

UroToday.com Adrenal and Retroperitoneum Section

 

From http://www.urotoday.com/57/browse_categories/adrenal_and_retroperitoneum/laparoscopic_management_
of_adrenal_lesions_larger_than_5_cm_in_diameter__abstract01112010.html

Trying something new…again

I saw this on another site and I stayed up all night making one for Cushies because I thought it was so cool.

 

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2. No Adware Policy : exposure to unwanted advertisements is not required in order to use the toolbar. The toolbar does not launch pop-up or pop-under advertisement windows or any other type of obtrusive ads.
3. Unobtrusive: The toolbar does not enable other applications to access data stored on your computer's hard drive or in your online accounts. The toolbar does not modify pages you visit or modify your search experience. You may voluntarily opt to receive Publisher notifications (such as Community Alerts) or use other advanced functionalities offered by cushie tools.
4. Easy uninstall : you can easily uninstall the toolbar at any time using the toolbar's standard uninstall package (Add/Remove Programs in Windows, Add-on Removal in Firefox, etc.).
5. Easy deactivation: you can easily deactivate your toolbar at any time by clicking the "View" menu in your browser and deselecting the name of your community toolbar.
6. Full control : you have full control over your toolbar and you can add/remove toolbar components at any time using your toolbar's Options dialog box.

7. Report - cushie tools is committed to ensuring your Privacy and safety while using your community toolbar. If you have a reason to believe that your rights have been infringed upon, please email privacy@conduit.com to contact the owners of Platform that was used by cushie tools to create your community toolbar, and your application will be handled at the earliest convenience.

Finally, I would like to add that installing this toolbar is possibly a way for the sites to make a little money although the hosting site doesn’t disclose how much they give back and how many people have to do how much searching to make any kind of profit.

 

The theory is that Google pays the host company, Conduit, like it does for Google ads – I’ve seen them on other sites but have never used them because I want to try to keep the sites ad free and non-tacky. Then, depending on the number of people who have installed this toolbar, and how much they use it, a percentage of that money is supposed to come back to Cushing’s Help.

 

I have no expectations of making any money, though. I just thought that it looked like an interesting new way for people to find things easily on the websites, listen to podcasts, and get the latest news.

 

Please note – after installation there’s a little popup window that says you might get alerts. I promise I won’t send those out unless it’s something serious like the boards are back up after a day of being down.

 

Thanks for reading! I hope you’ll give this a try.

Addison's Blog Alerts

Training because I can! Addison's disease, exercise and living in ...
By Dusty
Training because I can! Addison's disease, exercise and living in Idaho. Image Hosted by ImageShack.us ... Addison's Support, there's more than just this blog. Addison's Support the links · Addison's Support the Forum ...
Training because I can! Addison's... - http://addisonssupport.blogspot.com/

 

 

Addison Disease - Professional Medical Resources
Profession medical resources for "Addison Disease" including "Addison Disease: eMedicine Endocrinology", "Addison Disease: eMedicine Dermatology", ...

Addison Disease: eMedicine Endocrinology

is more than 2-fold higher in patients with Addison disease. Cardiovascular malignant and infectious diseases are responsible for the higher mortality rate. ... is seen in association with hyperpigmentation in idiopathic autoimmune Addison disease. It is due to the autoimmune destruction of melanocytes. ... http://emedicine.medscape.com/article/116467-overview

 

Addison Disease: eMedicine Dermatology

Addison disease in developing countries. Currently in developed countries Addison disease most commonly results from nonspecific autoimmune destruction of the adrenal gland. ... Addison disease Addison's disease primary adrenal insufficiency chronic adrenal insufficiency hypoadrenalism polyglandular autoimmune diseases polyglandular autoimmune disease I PGAD I polyglandular autoimmune ... http://emedicine.medscape.com/article/1096911-overview

Development of a Disease-Specific Quality of Life Questionnaire in Addison's Disease

Kristian Løvås*, Suzanne Curran, Marianne Øksnes, Eystein S. Husebye, Felicia A. Huppert,  and V. Krishna K. Chatterjee

 

Department of Medicine (K.L., S.C., V.K.K.C.), University of Cambridge, Cambridge CB2 0QQ, United Kingdom; Institute of Medicine (K.L., M.Ø., E.S.H.), University of Bergen, 5020 Bergen, Norway; Department of Medicine (K.L., E.S.H.), Haukeland University Hospital, 5021 Bergen, Norway; and Department of Psychiatry (F.A.H.), University of Cambridge, Cambridge CB2 2QQ, United Kingdom

 

* To whom correspondence should be addressed. E-mail: Kristian.lovas@helse-bergen.no.

 

Context: Patients with Addison's disease reproducibly self-report impairment in specific dimensions of general well-being questionnaires, suggesting particular deficiencies in health-related quality-of-life (HRQoL).

 

Objective: We sought to develop an Addison's disease-specific questionnaire (AddiQoL) that could better quantify altered well-being and treatment effects.

 

Design, Setting, Patients, Intervention, and Outcomes: We reviewed the literature to identify HRQoL issues in Addison's disease and interviewed patients and their partners in-depth to explore various symptom domains. A list of items was generated, and nine expert clinicians and five expert patients assessed the list for impact and clarity. A preliminary questionnaire was presented to 100 Addison's outpatients; the number of items was reduced after analysis of the distribution of the responses. The final questionnaire responses were assessed by Cronbach's {alpha} and Rasch analysis.

 

Results and Interpretation: Published studies of HRQoL in Addison's disease indicated reduced vitality and general health perception and limitations in physical and emotional functioning. In-depth interviews of 14 patients and seven partners emphasized the impact of the disease on the emotional domain. Seventy HRQoL items were generated; after the expert consultation process and pretesting in 100 patients, the number of items was reduced to 36. Eighty-six patients completed the final questionnaire; the responses showed high internal consistency with Cronbach's {alpha} 0.95 and Person Separation Index 0.94 (Rasch analysis).

 

Conclusions: We envisage AddiQoL having utility in trials of hormone replacement and management of patients with Addison's disease, analogous to similar questionnaires in GH deficiency (AGHDA) and acromegaly (AcroQoL).

 

 

From http://jcem.endojournals.org/cgi/content/abstract/jc.2009-1711v1

(Addison’s) A piece of presidential history solved the puzzle

By Sandra G. Boodman
Special to the Washington Post
Tuesday, December 15, 2009

 

As she lay in a heap, trying to figure out how badly she had hurt herself falling headfirst down a flight of stairs in the middle of the night, Rebecca Woodings grasped just how sick she really was.

 

For months doctors had been ratcheting up the medicines used to treat her intractable allergies. At one point she was taking 10 drugs a day and getting allergy shots. An economist who works for a large Washington law firm, Woodings, 49, told doctors she was tired; she assumed her fatigue was a consequence of her allergies, which were also causing a persistent cough. She did not tell them she was so exhausted she had to sit on the sidewalk while waiting for a bus and couldn't stand long enough to cook a meal.

 

Hours before she tumbled down the stairs of her Takoma Park home last June, an astute pulmonologist had figured out what was wrong -- and it had nothing to do with her lungs. That night, as Woodings tried to move the wrist she had broken in the fall, she focused on her 6-year-old son, realizing that if she had smacked her head she could have died. "I kept thinking, what would have happened to my child?"

 

In the fall of 2008 Woodings began feeling unusually tired. Walking less than a mile to the Metro in the morning made her break into a sweat. "It was very tiring," she said, and she recalled feeling puzzled. "I'm not terribly out of shape and I'm not overweight." Once on the Metro, Woodings made sure to get a seat; standing for 20 minutes was unthinkable.

 

During her annual checkup in November, her long-time internist at George Washington University discovered a Vitamin D deficiency and prescribed a short course of high-dose supplements.

 

By December, the fatigue was worse. Woodings had to sit down in the middle of a hymn during a church service. "All these little white-haired people around me are standing, and I couldn't," she recalled. When she mentioned the incident to a friend who works at the National Institutes of Health, she was told the symptoms sounded like a heart attack. Alarmed, Woodings immediately headed to a nearby emergency room, where an EKG and a chest X-ray showed that her heart was fine and her lungs were clear. Her father, a retired physician, suggested that maybe an antihistamine was causing her fatigue. Woodings stopped taking it and felt slightly more energetic.

 

By February, she was forced to sleep propped up on pillows and was taking a prescription cough syrup, which had little effect. The mother of a typically energetic kindergartner, she had started falling into bed around 8:30, when her son did. One night, she was so tired she told him to put himself to bed and crawled into bed at 8. Her allergist began administering allergy shots, which didn't help. Another doctor -- not her regular internist -- suggested she cut back on her sleep and get more exercise. Woodings replied that she was so tired she worried she might fall off a treadmill.

 

Routinely she arrived at the office at 9 a.m., already worn out. "It's really difficult to talk about being exhausted at a law firm," she said. "It sounds wimpy," so she didn't mention it.

 

In March, when she was handed a demanding new assignment with multiple deadlines, two new symptoms surfaced: Woodings began retching unpredictably -- "that damn cough," she remembered thinking -- and developed ferocious leg cramps at night. By then she noticed another peculiarity: Although she literally could not stand long enough to wait for a light to change while crossing the street, she could manage if she kept moving, walking slowly in a circle.

 

In early April, she went back to the allergist. He diagnosed a bad sinus infection and doubled the medications she was taking to 10 per day, including a short course of prednisone, a corticosteroid sometimes used to treat severe sinus infections.

 

After the first day, Woodings said, she felt markedly better. A week later the cough had disappeared and her energy slowly returned.

 

But by Memorial Day the fatigue was back and Woodings realized her problem wasn't allergies. She had stopped taking the allergy drugs, deciding that they might be the cause; her cough was gone. Woodings called her internist, whom she had not seen in six months. The doctor was heading out of town and Woodings decided to wait until her return rather than see a covering physician. In the meantime her physician ordered several tests, including those for Lyme disease and HIV, as well as CT scans of her lungs and sinuses.

 

On June 5, Woodings was told she had a possible bacterial infection in her lungs -- but not tuberculosis -- and was referred to GWU pulmonologist Susan Hasselquist. When she called to make an appointment, she was told that Hasselquist's first opening was a month away.

 

Desperate, Woodings decided to lie. "I said, 'I can't wait. The potential diagnosis is active TB.' " She was given an appointment for the next day.

 

On June 10 Woodings met with Hasselquist, who listened intently as Woodings recounted the events of the previous seven months. Unable to obtain a blood pressure using an automated cuff, Hasselquist measured it manually and found it was an alarmingly low 90/55. The lung specialist recalled being struck by how weak Woodings was: She lay down on the examining table while they talked because sitting up was too tiring. Hasselquist said she kept thinking of, and discarding, possible diagnoses. "I knew if we just kept talking I'd figure it out," she said.

 

Her eureka moment occurred when she zeroed in on Woodings's deep tan and asked her about it. Woodings, who is normally very fair, said that other people had remarked on it and that she hadn't spent much time in the sun.

 

Suddenly, Hasselquist said, she was certain what was wrong, a hunch triggered by photographs she'd seen of a ruddy-looking President John F. Kennedy, who had Addison's disease, a rare endocrine disorder that occurs when the adrenal glands become damaged and fail to produce enough cortisol and aldosterone, hormones vital for metabolic function. Most cases are the result of an autoimmune attack in which the immune system slowly destroys the adrenal glands. Woodings's dramatic improvement while taking prednisone, the steroid prescribed to treat her sinus infection, was a vital clue: It is one of the medicines used to treat Addison's.

 

Kennedy received an Addison's diagnosis at age 30; his sister, the late Eunice Kennedy Shriver, is also believed to have suffered from the disorder, which affects one to four of every 100,000 people, according to the National Institute of Diabetes and Digestive and Kidney Diseases.

 

Woodings had the classic symptoms of Addison's: progressive fatigue, muscle weakness, low blood pressure that falls further during a change in positions, and hyperpigmentation, which resembles a dark tan. The retching and legs cramps are also symptoms, although her allergies and cough are not.

 

Hasselquist did not mention her suspicion to Woodings because it would require confirmation from an endocrinologist. She said she suggested hospitalizing Woodings because she was so weak. When Woodings declined, Hasselquist warned her against standing up too quickly, which could cause dizziness.

 

After the appointment with Hasselquist, Woodings went straight home, ordered a pizza and went to bed. She awoke several hours later and headed for the bathroom to urinate. She remembers feeling dizzy, and then realizing she was at the bottom of the stairs, her wrist shattered. She managed to get up, call 911 and wake her son. Doctors in the ER set her wrist, told her to see an orthopedist because she would need surgery, then sent her home.

 

A few days later GWU endocrinologist Michael Irwig, to whom Hasselquist referred Woodings, confirmed the Addison's diagnosis. He prescribed prednisone and another drug Woodings will have to take for the rest her life to replace the hormones her body no longer produces.

 

Within a few weeks, Woodings said, she felt much better. Her energy level returned to normal, as did her blood pressure. Her tan is fading, and surgery on her wrist was successful.

 

"I can't fault any of the doctors," Woodings said, adding that she believes she should have called her internist early on, instead of consulting other physicians. "I think I could have described my condition a little better. I never said, 'I have to sit on the sidewalk waiting for a bus,' but rather, 'I'm tired all the time.' "

 

If you have a Medical Mystery that has been solved, e-mail medicalmysteries@washpost.com. To read previous mysteries, go to http://www.washingtonpost.com/health.

 

From http://www.washingtonpost.com/wp-dyn/content/article/2009/12/14/AR2009121402863.html

 

~~~~~~~~~~~~~~~

 

Feedback:

Woman's tale of Addison's disease proves the value of primary-care physicians

 

Tuesday, December 22, 2009

 

Of primary importance

 

"A piece of presidential history solved the puzzle" [Dec. 15], about the lady found to have Addison's disease, points out how crucial it is to have a primary-care focus in evaluating patients. Often patients scramble through a maze of specialists, as she did, without a strong primary-care clinician coordinating care.

 

My hope is that health reform will recognize the essential perspective that primary-care physicians from family medicine, geriatrics and internal medicine bring to patient care. They can save patients and the health-care system heartache and money. It is the most challenging field in medicine and the most holistic.

 

Our system should provide incentives for new medical school graduates to join these fields and reward these physicians appropriately. These doctors work very, very hard. Good primary-care clinicians are worth their weight in gold.

 

Christine Butler

Coordinator, Palliative Care Service

Sibley Memorial Hospital

Washington

 

From http://www.washingtonpost.com/wp-dyn/content/article/2009/12/18/AR2009121803706.html

Diagnosis and Treatment of Adrenal Tumors: A Single-Center Experience with 238 Cases

Abdul-Monem Gomha, Yasser Osman, Mohsen El-Mekresh, Mohamed Abou El-Ghar, Ibrahim Eraky
Urology Department, Urology and Nephrology Center, Mansoura University, Mansoura, Egypt

Address of Corresponding Author

Urol Int 2009;83:433-437 (DOI: 10.1159/000251184)

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 Key Words

• Adrenal mass
• Adrenalectomy

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 Abstract

Objective: It was the aim of this study to review and analyze clinical data on the diagnosis and management of patients with adrenal masses.

 

Patients and Methods: Between 1976 and 2005, 238 patients admitted to our institute with adrenal masses were reviewed. Incidence, clinical features, imaging technique findings, surgical approaches, morbidity and mortality, as well as pathological diagnoses were reported.

 

Results: The series comprised 134 males and 104 females (mean age 33.3 ± 20.3 years). Right-sided masses were more common (63.4%), with a mean size of 7.7 ± 4 cm. Pain was the most frequent presenting symptom (53.4%), while 62 (26%) had a functional tumor. Incidentaloma was diagnosed in 49 patients (20.6%). Both computed tomography and magnetic resonance imaging showed a high diagnostic yield (sensitivities of 98.9 and 100%, respectively). Open adrenalectomy was performed in 153 patients (64.3%), while a laparoscopic approach was employed in 53 patients (22.3%). The intraoperative complication rate was 14.7%, the postoperative complication rate 6.1% and perioperative mortality 1.7%. Most of the excised masses were pheochromocytomas (26.4%). Conclusions: Computed tomography is recommended as the first diagnostic modality to define and characterize adrenal masses. Laparoscopic adrenalectomy is currently replacing open surgery as the standard surgical management of adrenal masses.

Copyright © 2009 S. Karger AG, Basel

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 Author Contacts

Yasser Osman, MD
Urology and Nephrology Center
Mansoura University
Mansoura (Egypt)
Tel. +20 50 226 2222, Fax +20 50 226 3717, E-Mail y_osman99@yahoo.com

---

 Article Information

Received: October 20, 2008
Accepted: December 9, 2008
Published online: December 08, 2009
Number of Print Pages : 5
Number of Figures : 1, Number of Tables : 3, Number of References : 20

 

From http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowAbstract&ArtikelNr=251184&Ausgabe=253627&ProduktNr=224282

Epidemiology of adrenal crisis in chronic adrenal insufficiency – the need for new prevention strategies

European Journal of Endocrinology (2009) In press
DOI: 10.1530/EJE-09-0884
Copyright © 2009 by European Society of Endocrinology

Stefanie Hahner, Melanie Loeffler, Benjamin Bleicken, Christiane Drechsler, Danijela Milovanovic, Martin Fassnacht, Manfred Ventz, Marcus Quinkler and Bruno Allolio

S Hahner, Endocrinology and Diabetes Unit, University of Wuerzburg, Wuerzburg, D-97080 , Germany
M Loeffler, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany
B Bleicken, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
C Drechsler, Dept. of Nephrology, University of Würzburg, Würzburg, Germany
D Milovanovic, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany
M Fassnacht, Dept. of Medicine I, University of Würzburg, Würzburg, Germany
M Ventz, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
M Quinkler, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
B Allolio, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany

 

Correspondence: Stefanie Hahner, Email: hahner_s@medizin.uni-wuerzburg.de

 

Objective: Adrenal crisis (AC) is a life-threatening complication of adrenal insufficiency (AI). Here we evaluated frequency, causes and risk factors of AC in patients with chronic AI.

Methods: In a cross-sectional study 883 patients with AI were contacted by mail. 526 patients agreed to participate and received a disease specific questionnaire.

 

Results: 444 data sets were available for analysis (primary adrenal insufficiency, PAI n=254, secondary adrenal insufficiency, SAI n=190). 42% (PAI 47%, SAI 35%) reported at least one crisis. 384 AC in 6092 patient years were documented (frequency of 6.3 crises/100 patient years). Precipitating causes were mainly gastrointestinal infection and fever (45%) but also other stressful events (e.g. major pain, surgery, psychic distress, heat, pregnancy). Sudden onset of apparently unexplained AC was also reported (PAI 6.6%, SAI 12.7%). Patients with PAI reported more frequent emergency glucocorticoid administration (42.5% vs 28.4%, p=0.003)) Crisis incidence was not influenced by educational status, BMI, glucocorticoid dose, DHEA treatment, age at diagnosis, hypogonadism, hypothyroidism or growth hormone deficiency. In PAI, patients with concomitant non-endocrine disease were at higher risk of crisis (OR=2.02, 95% CI 1.05-3.89, p=0.036). In SAI, female sex (OR=2.18, 95%CI 1.06-4.5, p=0.035) and diabetes insipidus (RR=2.71, 95%CI 1.22-5.99, p=0.014) were associated with higher crisis incidence.

 

Conclusion: AC occurs in a substantial proportion of patients with chronic AI, mainly triggered by infectious disease. Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI.

 

From http://www.eje.org/cgi/content/abstract/EJE-09-0884v1